Lim_ NKT细胞淋巴瘤生物学和治疗的最新进展.pptx

ManagementofExtranodalNK/Tcelllymphoma(ENKL)

SoonThyeLim,MD

Head,DepartmentofMedicalOncology

NationalCancerCentreSingapore

Duke-NationalUniversitySingaporeMedicalSchool

Content

.EpidemiologyandGeneticsofENKL

.ClinicalPresentation

.PrognosticFactors

.ManagementofearlystageENKTL

.ManagementofadvancedENKTL

–RoleofconsolidationHaematopoieticstemcelltransplant(HSCT)

.NovelagentsinManagementofRelapsed/RefractoryENKL

Presentedby:LimSoonThye

EpidemiologyandGeneticsofExtranodalNK/TCellLymphoma(ENKL)

Presentedby:LIMSoonThye

ExtranodalNK/TCellLymphoma(ENKL):DistinctGeographicalDistribution

•ENKLIsmoreprevalentinAsiancountriesandpartsofLatin

Population-basedstudy:UsingdatafromtheHongKong(HK)CancerRegistryandUnitedStates(U.S.)SEERProgram

BassigBAetal.CancerEpidemiology2016

•HigherratesofENKLinHKcomparedtoU.S.

AsiansandWhitesfor

bothmalesandfemales

AmericacomparedtotheWest

Presentedby:LimSoonThye

StrongassociationwithEbsteinBarrVirus(EBV)

EBV-encodedRNA(EBER)in-situhybridization(ISH)isinvariablypositive.

EBVGeneexpressionpattern

%EBVpositivity

Presentedby:LimSoonThye

LatencyII

LatencyII

EBER-ISH

>90%

>95%

T/NK

ENKL

AITL

OR(95%回CI)

2.65回(2.08-3.37)

1.61(1.12-2.33)

1.73回(1.22-2.46)

1.52回(1.17-1.98)

1.45回(1.03-2.05)

1.56回(1.33-1.84)

1.84回(1.61-2.11)

GeneticsusceptibilitytoENKL?

.Duetoitsuniqueclinicalfeatures,geographicaldistributionandassociationwithEBV:

–WehypothesizedthatindividualsusceptibilitytoENKLcouldhaveahereditablepredisposition

.PerformedthefirstGWASforENKLon189ENKLcasesand957controls.Replicationwasconductedinfourindependentpatientseries

ReplicationGuangdong1

DiscoveryGuangdong

OverallAll回samples

StagesCollection

Presentedby:LimSoonThye

Replication回meta-an

RAF回controls